Clinical Manifestations of Sjögren's Syndrome
Sjögren's syndrome primarily presents with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) due to lymphocytic infiltration of lacrimal and salivary glands, along with systemic manifestations including fatigue, arthralgia, myalgia, and potential involvement of multiple organ systems. 1
Key Ocular Manifestations
Dry eyes with symptoms of:
- Irritation, itching, or pain
- Foreign body sensation
- Light sensitivity (photophobia)
- Blurry vision or unexplained vision changes
- Frequent use of eye drops for dryness 1
Objective findings on examination:
- Decreased tear film stability (reduced tear break-up time)
- Reduced tear meniscus height
- Punctate epithelial erosions with fluorescein staining
- Abnormal ocular staining score (≥5) on lissamine green or rose bengal staining
- Schirmer test ≤5 mm/5 minutes (indicating decreased tear production) 1
Key Oral Manifestations
Dry mouth with symptoms of:
- Need for liquids to swallow dry foods
- Frequent sipping/drinking of water
- Burning sensation in the mouth
- Angular cheilitis (painful sores at corners of mouth)
- Frequent dental cavities, particularly at gumline
- Teeth that chip, crack, or erode
- Gum inflammation or recession 1
Objective findings:
- Unstimulated salivary flow rate ≤0.1 ml/minute
- Enlargement of parotid and/or submandibular glands 1
Systemic Manifestations
Musculoskeletal
- Arthralgia (joint pain) without significant inflammation
- Myalgia (muscle pain)
- Arthritis resembling rheumatoid arthritis in some cases 1
Fatigue and Neurological
- Extreme fatigue (affects quality of life significantly)
- Peripheral neuropathy (numbness, altered sensation, burning pain in extremities)
- Cognitive impairment affecting memory, executive function, and attention 1, 2
Vascular
- Raynaud's phenomenon (fingers turning pale/blue in cold) 1
Pulmonary
- Chronic dry cough (present in ~38% of patients)
- Xerotrachea (dry trachea)
- Small airway disease (bronchiolitis)
- Bronchiectasis
- Interstitial lung disease 1
Dermatological
- Xerosis (dry skin)
- Pruritus
- Cutaneous vasculitis (in severe cases) 3
Genitourinary
- Vaginal dryness
- Painful intercourse 1
Hematological/Immunological
- Lymphadenopathy
- Increased risk of lymphoproliferative disorders (especially in patients with decreased C4 levels) 1, 3
Diagnostic Criteria
Sjögren's syndrome classification criteria include a weighted sum of five items:
- Anti-SSA/Ro antibody positivity (3 points)
- Focal lymphocytic sialadenitis with focus score ≥1 foci/4 mm² (3 points)
- Abnormal ocular staining score ≥5 (1 point)
- Schirmer's test result ≤5 mm/5 minutes (1 point)
- Unstimulated salivary flow rate ≤0.1 ml/minute (1 point)
A total score of ≥4 meets the criteria for primary Sjögren's syndrome 1.
Primary vs. Secondary Sjögren's
- Primary Sjögren's syndrome: Occurs alone without other autoimmune conditions
- Secondary Sjögren's syndrome: Occurs in association with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma 1, 3
Epidemiology
- Prevalence approximately 0.4%
- Annual incidence of 3.9-5.3 per 100,000
- Female-to-male ratio of 20:1
- Most commonly diagnosed in middle-aged women 1, 4
Clinical Pearls and Pitfalls
- About 10% of patients with clinically significant dry eye have underlying Sjögren's syndrome
- Many patients are undiagnosed at the time they present to ophthalmology with dry eye complaints
- Patients with Sjögren's syndrome should be co-managed with a rheumatologist due to potential systemic complications
- Patients are at increased risk for lymphoma development, particularly those with decreased C4 levels
- Extraglandular manifestations may develop in approximately one-third of patients 1, 4
Early recognition of the diverse clinical manifestations of Sjögren's syndrome is crucial for timely diagnosis and management to prevent complications and improve quality of life.